Infant Botulism

Audience Emergency medicine and pediatric residents, and pediatric emergency medicine (PEM) fellows. Introduction Botulism is a rare but serious cause of infant hypotonia, vomiting, and respiratory failure. The differential diagnosis and management of a hypotonic infant with progressive weakness leading to respiratory failure is a rare presentation with high morbidity and mortality.1 Infants with botulism generally present with vague complaints that progressively worsen over time.2 Recognition of descending paralysis in an infant as well as signs of respiratory failure are key to preventing an adverse outcome. A key component of botulism treatment is recognizing the need to mobilize local resources to obtain BabyBIG® (botulism immune globulin). This process can and should begin in the emergency department. Educational Objectives After this simulation learners should be able to: 1) develop a differential diagnosis for the hypotonic infant, 2) recognize signs and symptoms of infant botulism, 3) recognize respiratory failure and secure the airway with appropriate rapid sequence intubation (RSI) medications, 4) initiate definitive treatment of infant botulism by mobilizing resources to obtain antitoxin, 5) continue supportive management and admit the patient to the pediatric intensive care unit (PICU), 6) understand the pathophysiology and epidemiology of infant botulism, 7) develop communication and leadership skills when evaluating and managing critically ill infants. Educational Methods This simulation case was performed using a high-fidelity Laerdal SimBaby with intubating capabilities and real-time vital sign monitoring. Additionally, this case can be performed with low fidelity manikins with supplemental scripting and visual stimuli. With minor adjustments, this case could be modified into an oral boards case. Research Methods We obtained feedback from a convenience sample of random participants after the simulation case and debrief were completed. The sample of emergency medicine residents (N=21) and PEM fellow (N=1) completed 5 questions on a 5-point Likert scale. Results The emergency medicine residents and PEM fellow had mostly favorable feedback regarding the simulation and debriefing. Most strongly agreed or agreed that this would improve their performance in an actual clinical setting. Discussion Infant botulism is a rare condition, presenting as vague non-specific complaints that worsen over time. It is important to differentiate infant botulism from other causes of weakness, hypotonia, and respiratory failure. This case presents learners with a high acuity, rare case of infant botulism and allows them to work through a complex pediatric patient encounter in a psychologically safe space. The presence of a standardized patient to play the patient’s parent is key to assess learners’ nontechnical communication skills and to increase fidelity during the simulation. Topics Infant botulism, pediatric emergency medicine, respiratory failure, hypotonia, toxicology.


Linked objectives and methods:
Infant botulism is a rare syndrome that has significant morbidity and mortality if not recognized and promptly treated. 1 Cases of infant botulism present as progressively worsening vague symptoms that may include decreased feeding, hypotonia, and vomiting. 2 It is important for the emergency medicine physician to be able to diagnose and appropriately treat infant botulism. This simulation will challenge the learners to obtain a thorough history and perform a physical exam to develop a differential diagnosis for hypotonia in an infant (Objective 1) and recognize infant botulism through key points in the history and physical (Objective 2). As the learners pursue a workup for this patient, they should be able to manage the patient's symptoms of hypoxia and worsening respiratory failure, ultimately intubating the patient (Objective 3). Learners should initiate definitive treatment of infant botulism by mobilizing resources to obtain antitoxin (Objective 4). Once the diagnosis of infant botulism is made and the patient's airway is secure, the learners should be able to continue supportive management and admit the patient to the pediatric intensive care unit (PICU) (Objective 5). Through the course of this simulation and debrief, learners will better understand the pathophysiology and epidemiology of infant botulism (Objective 6) and develop communication and leadership skills when evaluating and managing critically ill infants (Objective 7). Topics: Infant botulism, pediatric emergency medicine, respiratory failure, hypotonia, toxicology.

Objectives:
At the conclusion of this simulation, learners will be able to: 1. Review the differential diagnosis for the hypotonic infant 2. Recognize signs and symptoms of infant botulism 3. Recognize respiratory failure and secure the airway with appropriate RSI medication and equipment 4. Understand importance of mobilizing resources to procure botulism immune globulin 5. Provide appropriate supportive care and disposition 6

Case Description & Diagnosis (short synopsis):
The patient is a 2-month-old male with no past medical history or significant birth history presenting to a community emergency department with hypotonia, decreased oral intake, drooling, and constipation. He presents to the emergency department with his parent. The parent states that prior to this presentation, the patient had been fussy and colicky and if asked, will admit they gave him a honey pacifier to soothe him. The learner should perform a primary survey to assess the patient. The learner should recognize significant secretions and hypotonia and the need to protect the patient's airway. The goal of this case is to recognize infant botulism as a rare cause of hypotonia in infants and mobilize appropriate resources to provide treatment. Learners will care for a critically ill infant and transfer them to the PICU. Background and brief information: The scenario takes place in a community emergency department. A parent brings their 2-month-old infant with a chief complaint of poor feeding.

Initial presentation:
The patient has come from home by private vehicle and has been triaged by nursing as an ESI (emergency severity index) level 2. The patient is a 2-month-old male with no past medical history or significant birth history presenting with hypotonia, decreased oral intake, drooling, and constipation.
How the scene unfolds: The patient is a 2-month-old infant who is laying on the gurney in no acute distress. The parent is present and is intermittently wiping drool from the patient's mouth, simulated by water soluble lubricant. On primary survey, learners should assess the patient's airway, breathing, and circulation and obtain a full set of vitals. Vital signs are notable for tachycardia, decreased respiratory rate and hypoxia. The learner is expected to place the patient on supplemental oxygen to treat the patient's hypoxia and use suction to support the airway. Learners should obtain a history from the patient's parent, including feeding and stooling habits. The parent states that over the past few days their child has had poor feeding and has had decreased number of stools and more difficulty with stooling. Prior to this, the parent states that their baby had been fussy and to soothe him, they gave him a honey pacifier. This information should be given if learners ask about changes in diet or new ingestion in the past week. If history of honey pacifier use is not obtained, then the nurse can prompt for any changes in diet or ingestion. The patient has not taken a full bottle in over 24 hours due to lethargy and has not been waking up for feeds. The parent states that today the infant has not been as active as normal and has been drooling. Learners should perform a physical exam. The physical exam is notable for mydriasis, bilateral ptosis, and hypotonia. The infant mannikin Laerdal SimBaby can simulate ptosis and mydriasis, visual stimulus can be given to replace these findings in low fidelity simulation or high-fidelity pediatric mannequin without these capabilities. The patient will be hypotonic, drooling, with ptosis, and high endtidal CO 2 . Learners should place an IV and obtain laboratory studies. Learners should obtain a finger-stick blood glucose (FSBG) in the first five minutes and give IV glucose to treat hypoglycemia. If FSBG is not checked, then nursing will prompt learners to check. The learners should recognize the need to intervene on the patient's airway and perform rapid sequence intubation, regardless of seizure activity. The learners should pick equipment appropriate for the patient's age, weight, and height and appropriately dose medications. If the parent is not included and updated throughout the resuscitation, then the parent will ask clarifying questions. Once the intubation is performed and the airway is secure, a CT brain and lumbar puncture should be considered as a part of an infectious and altered mental status work-up for 55 this age. Because this case will occur in a community emergency department, learners will transfer the patient to a pediatric center. PICU admission is the only appropriate disposition for this patient. The treatment for botulism, BabyBIG ® , is only available in California and must be mobilized through the health department. If the health department has not been contacted prior to admission to PICU, the accepting physician will mention obtaining BabyBIG ® . History: • History of present illness: The patient is a 2-month-old male presenting with hypotonia, decreased oral intake, drooling, and constipation. The parent states that over the past few days their child has had poor feeding and has had decreased number of stools and more difficulty with stooling. Prior to this, the parent states that their baby had been fussy and to soothe him they gave him a honey pacifier. The patient has not taken a full bottle in over 24 hours and has not been waking up for feeds. The parent states that today, the infant has not been as active as normal and has been drooling. Obtain basic history and perform secondary exam Learners should obtain history including feeding amount and frequency, stooling frequency, and activity level. If no details are obtained, the nurse will prompt with questions for the parent.
Nurse prompt: Are you giving anything besides breast feeding? Parent will admit to using honey pacifier to soothe the patient. The patient should be transferred and admitted to the pediatric intensive care unit for continued management.
If participants do not initiate treatment from the ED, then the ICU physician will ask about calling the health department.

Differential Diagnosis of Hypotonia in Infants:
Botulism, SMA (Spinal Muscular Atrophy) type 1, Metabolic disorder (Leigh's), brainstem encephalitis, stroke, myasthenia gravis, neuromuscular disease, sepsis, drug ingestion, Guillain-Barre, Lambert-Eaton syndrome. The distinguishing feature between botulism and other pathologies causing hypotonia is the history of spore ingestion and the physical exam. Myasthenia gravis in the newborn also presents with hypotonia and bulbar symptoms leading to poor suck and increased secretions; however, ptosis and ocular symptoms are less prevalent. Hypermagnesemia can present with generalized hypotonia; this will be diagnosed by lab results. Guillain-Barre is typically an ascending paralysis with absent reflexes. Sepsis and encephalitis in the newborn will present with fever and preceding infectious symptoms. In SMA Type 1, generalized hypotonia is present, but upper cranial nerves are typically spared, and onset of symptoms is subacute to chronic. The key in diagnosing this case is clinical suspicion and history of botulinum spore ingestion.

Treatment: The treatment for infant botulism is intravenous botulism immune globulin (BabyBIG ® ). This can only be obtained from the California Department of Health Services Infant
Botulism Treatment and Prevention Program. High suspicion for infant botulism and early mobilization of resources is key because it takes time for immune globulin to be transported to the facility. BabyBIG ® binds the toxin and prevents symptoms from progressing and is effective for both type A and type B toxins. BabyBIG ® should be given prior to lab confirmation of botulism. However, it does not reverse current symptoms, so administration should be done as soon as possible. BabyBIG ® administration reduced mean hospital stay (2.6 weeks vs 5.7 weeks), reduced mean ICU stay, and has no serious adverse effects. A single dose will neutralize all C. botulinum toxin available for absorption in the body for at least 6 months. Supportive care is the mainstay of treatment in these patients. Most patients require intensive care and half require mechanical ventilation for respiratory failure. Of note, antibiotics are not recommended due to concern for lysis and increased toxin burden.
Treatment for hypoglycemia in infants depends on ability to swallow and IV access. If the patient is conscious and able to swallow safely, hypoglycemia can be treated with glucose gel, sweetened juice 0.3 g/kg goal of 10-20 g. If the patient is altered or unable to swallow, then IV glucose should be given. The goal dosage is 0.5 g/kg and volume and concentration depend on the patient's age. For infants and young children less than 5 years of age, 5 mL/kg of 10% dextrose solution (D10) should be administered. For patient's older than 5 years old, 2mL/kg of 25% dextrose solution (D25) should be administered. If the patient is unable to take oral glucose and IV access is unable to be obtained, consider IO insertion for access. However, glucagon can be given to treat hypoglycemia. The dosing is 0.5 mg for patients that weigh less than 25 kg and 1 mg for patients weighing greater than 25 kg.

DEBRIEFING AND EVALUATION PEARLS
Rocuronium is the drug of choice for RSI in patients with botulism because the paralytic is nondepolarizing. Use of succinylcholine can cause lethal hyperkalemia in patients with botulism, due to depolarization. Impaired acetylcholine release due to botulism toxin leads to upregulation of acetylcholine receptors and subsequent depolarization leads to potassium efflux and hyperkalemia.
Intubation equipment size is important to consider in pediatric patients. Broselow tape can be used to select equipment size and dosing of common resuscitation medications based on height. A two-month-old should be intubated using a Miller 1 blade.